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1.  Maltoma of Thyroid: A Rare Thyroid Tumour 
Case Reports in Otolaryngology  2013;2013:740241.
Introduction. Primary thyroid lymphomas constitute up to 5% of all thyroid malignancies and can be divided into non-Hodgkin's lymphomas (NHLs) of B- and T-cell types, as well as Hodgkin's lymphomas. Mucosa-associated lymphoid tissue (MALT) lymphomas are a relatively recently recognized subset of B-cell NHLs, and they are listed as extranodal marginal zone lymphomas according to the revised European-American lymphoma classification. Case Report. We report an uncommon case of a 44-year-old man, who noted a painless, growing mass on right side of his neck of the three-month duration. Thyroid profile was within normal limits. FNAC showed lymphocytic thyroiditis. The patient underwent a right hemithyroidectomy. The histologic examination and the immunohistochemistry showed an extra nodal marginal B-cell type maltoma (malt lymphoma). CHOP chemotherapy with rituximab was given. The clinical course has been favourable in the first year of followup, with no evidence of local or systemic recurrence of the disease. Discussion. Marginal zone lymphoma encompasses a heterogeneous group of B-cell tumours that variously arise within the lymph nodes, spleen, or extranodal tissues. A case of maltoma of thyroid is presented for its rarity and diagnostic dilemmas. Conclusion. Maltomas are slow-growing lymphomas. The optimal treatment and followup of patients with thyroid maltomas remain controversial at present.
doi:10.1155/2013/740241
PMCID: PMC3582082  PMID: 23476858
2.  Primary extranodal lymphomas of stomach: clinical presentation, diagnostic pitfalls and management 
Annals of Oncology  2008;19(12):1992-1999.
Gastrointestinal lymphoma is the most common form of extranodal lymphoma, accounting for 30%–40% of cases. The most commonly involved site is the stomach (60%–75% of cases), followed by the small bowel, ileum, cecum, colon and rectum. The most common histological subtypes are diffuse large B-cell lymphoma (DLBCL) and marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT). Helicobacter pylori infection has been implicated in the pathogenesis of MALT gastric lymphoma, but its role in gastric diffuse large B-cell non-Hodgkin's lymphoma (NHL) is controversial. The therapeutic approach for patients with gastric NHL has been revised over the last 10 years. Conservative treatment with anthracycline-based chemotherapy alone or in combination with involved-field radiotherapy has replaced gastrectomy as standard therapy in cases with DLBCL. Additionally, MALT lymphomas are mainly treated with antibiotics alone, which can induce lasting remissions in those cases associated with H. pylori infection. Nevertheless, various therapeutic aspects for primary gastric lymphomas are still controversial and several questions remain unanswered. Among others, the role of rituximab, consolidation radiotherapy as well as H. pylori eradication in histological aggressive subtypes warrants better clarification.
doi:10.1093/annonc/mdn525
PMCID: PMC2733120  PMID: 18647965
diffuse large B-cell lymphomas; extranodal lymphomas; Helicobacter pylori infection; mucosa-associated lymphoid tissue; primary gastric lymphomas
3.  Gastric Marginal Zone B Cell Lymphoma of the Duodenum 
Case Reports in Gastroenterology  2011;5(3):578-582.
Small bowel lymphomas of the extranodal type occur in the young and are characteristically associated with malabsorption syndrome. We present the case of an elderly in whom there was no malabsorption and the duodenal tumor was a gastric type marginal zone B cell lymphoma also known as gastric mucosa-associated lymphoid tissue (MALT) lymphoma. A 73-year-old woman presented to the emergency room with 2 weeks of general weakness, recurrent vomiting containing food particles and abdominal distension. She had been diagnosed with diabetic gastroparesis 4 years prior. CT of the abdomen and pelvis was suggestive of gastric outlet obstruction but no evidence of pancreatic or duodenal mass. Endoscopy and biopsy of the tumor obstructing the distal first part of the duodenum confirmed a gastric marginal MALT lymphoma. The patient's symptoms improved with radiotherapy. Gastric MALT lymphoma, an extranodal lymphoma primarily described in the stomach, can also present in the small bowel and is not associated with malabsorption.
doi:10.1159/000331137
PMCID: PMC3219481  PMID: 22110418
Small bowel lymphoma; Gastric lymphoma; Duodenum
4.  Primary breast lymphomas: a multicentric experience 
Background
The Primary Breast Lymphomas (PBL) represent 0,38-0,70% of all non-Hodgkin lymphomas (NHL), 1,7-2,2% of all extranodal NHL and only 0,04-0,5% of all breast cancer. Most frequent PBLs are the diffuse large B cell lymphomas; in any case-reports MALT lymphomas lack or are a rare occurrence. Their incidence is growing. From 1880 (first breast resection for "lymphadenoid sarcoma" carried out by Gross) to the recent past the gold standard treatment for such diseases was surgery. At present such role has lost some of its importance, and it is matter of debate.
Methods
Twenty-three women affected by PBL underwent surgery. Average age was 63 years (range: 39-83). Seven suffered of hypothyroidism secondary to autoimmune thyroiditis. Fourteen patients underwent mastectomy, nine patients received quadrantectomy (average neoplasm diameter: 1,85 cm, range: 1,1-2,6 cm). In 10 cases axillary dissection was carried out. Pathologic examination revealed 16 diffuse large B cell lymphomas and 7 MALT lymphomas.
Results
Seven patients in the mastectomy group had a recurrence (50%), and all of them with diffuse large B cell lymphomas at stage II. Two of these had not received chemotherapy. No patient undergoing quadrantectomy had recurrence. In the mastectomy group disease free survival (DFS) at 5 and 10 years was 57 and 50%. Overall survival (OS) at 5 and 10 years was 71.4% and 57.1% respectively. All recurrences were systemic. DFS and OS at 5 and 10 years was 100% in the quadrantectomy group. In the patients with recurrence mortality was 85.7%. For stage IE DFS and OS at 5 and 10 years were 100%. For stage II DFS at 10 years was 62.5% and 56.2% respectively; OS at 5 and 10 years was 75% and 62.5% respectively. For MALT lymphomas DFS and OS at 5 and 10 years were 100%. For diffuse large B cell lymphomas DFS at 5 and 10 years was 62.5% and 56.2% respectively; OS at 5 and 10 years was 75% and 62,5% respectively.
Conclusions
The role of surgery in this disease should be limited to get a definitive diagnosis while for the staging and the treatment CT scan and chemio/radioterapy are repectively mandatory. MALT PBLs have a definitely better prognosis compared to large B cell lymphomas. The surgical treatment must always be oncologically radical (R0); mastectomy must not be carried out as a rule, but only when tissue sparing procedures are not feasible. Axillary dissection must always be performed for staging purposes, so avoiding the risk of under-staging II o IE, due to the possibility of clinically silent axillary node involvement.
doi:10.1186/1477-7819-8-53
PMCID: PMC2903594  PMID: 20584320
5.  Longlasting Remission of Primary Hepatic Mucosa-associated Lymphoid Tissue (MALT) Lymphoma Achieved by Radiotherapy Alone 
Primary hepatic lymphoma is a rare disorder representing less than 1% of all extranodal lymphomas. Histological examination of a primary hepatic lymphoma usually reveals a diffuse large B-cell lymphoma; there have been few reports of primary hepatic mucosa-associated lymphoid tissue (MALT) lymphomas. A 67-year-old man was being treated for a duodenal ulcer; while receiving therapy for the ulcer, a liver mass was incidentally found on abdominal ultrasonography. The pathologic diagnosis of the hepatic mass was an extranodal marginal zone B-cell lymphoma of MALT. The patient underwent radiotherapy with a total of 4,140 cGy delivered. The patient achieved complete remission and has been followed for 6 years with no recurrence of the disease. This report reviews the case of a primary hepatic extranodal marginal zone B-cell lymphoma of MALT successfully treated by radiotherapy alone.
doi:10.3904/kjim.2006.21.2.127
PMCID: PMC3890736  PMID: 16913444
Primary hepatic lymphoma; Mucosa-associated lymphoid tissue (MALT); Radiotherapy
6.  Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features 
Primary gastrointestinal lymphoma comprises 10-15% of all non-Hodgkin lymphomas and encompasses 30-40% of the total extranodal lymphomas. Approximately 60-75% of cases occur in the stomach, and then the small bowel, ileum, cecum, colon and rectum. Lymphoid neoplasms may consist of mature B, T and less commonly extranodal NK/T cells. Of these, the two most frequently encountered histologic subtypes are extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), where Helicobacter pylori infection is implicated in a number of cases, and diffuse large B cell lymphoma. Several B cell lymphomas are associated with chromosomal aberrations. Enteropathy-associated T cell lymphoma, type I in particular, usually arises in a background of celiac disease. T cell gene rearrangement confirms clonality. NK/T cell neoplasms are invariably associated with Epstein-Barr virus infection and are often aggressive; thus, differentiation from a benign NK-cell enteropathy is paramount. Although incidence of other hematopoietic malignancies in the gastrointestinal tract such as plasma cell myeloma associated with amyloidosis, plasmablastic lymphoma, Hodgkin disease, histiocytic sarcoma and mast cell sarcoma is extremely rare, these entities have been documented, with the latter two demonstrating aggressive clinical behavior. Endoscopic ultrasonography is an important adjunct in disease staging and follow-up. Conservative antibiotic treatment of stage I MALT lymphomas with associated Helicobacter pylori infection achieves good clinical outcome with high remission rate. Chemotherapy, radiation and rarely surgery are reserved for advanced diseases or cases resistant to conservative therapy and those not associated with Helicobacter pylori infection.
doi:10.3978/j.issn.2078-6891.2012.024
PMCID: PMC3418529  PMID: 22943012
Gastrointestinal lymphomas; MALT lymphoma; NK/T-cell enteropathy
7.  Marginal zone lymphoma: old, new, targeted, and epigenetic therapies 
Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5–17% of all non-Hodgkin’s lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. Treatment should be patient tailored and can range from a ‘watchful waiting’ approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Newer therapies such as bendamustine, everolimus, lenalidomide, vorinostat and phosphoinositide 3-kinase inhibitors are in clinical trials for patients with relapsed or refractory MZL and have shown promising results. We are presently conducting clinical trials testing the efficacy of the epigenetic activity of cladribine as a hypomethylating agent in combination with the histone deacetylase inhibitor (HDACi) vorinostat and rituximab in patients with MZL. Further studies with the newer agents should be done both in newly diagnosed or relapsed/refractory MZL to streamline the care and to avoid the use of toxic chemotherapies as initial treatment.
doi:10.1177/2040620712453595
PMCID: PMC3627321  PMID: 23616915
DNA methylation; epigenetics; marginal zone lymphoma; non-Hodgkin’s lymphoma; purine analogs
8.  Primary MALT Type Skin Lymphoma—Is ‘Wait and See’ a Possible Strategy? 
Clinical Medicine. Oncology  2008;2:153-158.
Primary cutaneous lymphomas are the second most common site of extranodal non-Hodgkin lymphoma. A specifically type named extranodal marginal zone B-cell lymphomas are indolent low-grade neoplasma.
We report a case of a 42-year-old white man with multiple subcutaneous tumors located on the trunk and neck. The histopathological exam showed a non-epidermotropic, dense lymphocytic infiltrate. Histologic, immunohistochemical and cytologenetic analysis diagnosed primary cutaneous B-cell lymphoma MALT type. Investigation for other extranodal MALT lymphoma gastrointestinal tract, lung, salivary and thyroid glands was negative. The patient refused radiotherapy, but he accepted every 6 months close follow-up. Over a seven years period, we noticed a progressively disappearance of the skin lesions.
The necessity of aggressive treatment of this disease with excellent prognosis is discussed.
The treatment necessity of primary cutaneous B-cell lymphoma MALT type is discussed.
PMCID: PMC3161667  PMID: 21892278
primary cutaneous B-cell lymphoma; MALT type; therapy
9.  A Case of Synchronous Lung Adenocarcinoma and Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT) Type 
Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (extranodal MZL) is a distinct subgroup of non-Hodgkin's lymphoma. Pulmonary extranodal MZL is a rare entity and accounts for less than 0.5% of primary pulmonary malignancies. Only a few cases of simultaneous occurrence of lung cancer and pulmonary extranodal MZL have been reported. A 60-year-old woman was referred to our hospital with a pulmonary nodule. She was diagnosed with lung adenocarcinoma by percutaneous needle biopsy. The protrusions into the left main bronchus were found by accident while performing bronchoscopy during lung cancer evaluation. The bronchial lesions were diagnosed as extranodal MZL. Although the patient underwent surgical resection for the lung adenocarcinoma, the pulmonary extranodal MZL was left untreated; it was monitored during follow-up visits. To our knowledge, this is the first report of synchronous lung adenocarcinoma and primary extranodal MZL of the main bronchus.
doi:10.4046/trd.2012.73.1.61
PMCID: PMC3475473  PMID: 23101026
Lymphoma, B-Cell, Marginal Zone; Adenocarcinoma of Lung; Lung Neoplasms
10.  Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases 
Journal of Clinical Pathology  2000;53(6):458-461.
Aim—To report the clinical and histological features and outcome of primary and secondary malignant lymphomas of the urinary bladder.
Methods—Eleven cases of malignant lymphoma of the urinary bladder were obtained from the registry of cases at St Bartholomews and the Royal London Hospitals. The lymphomas were classified on the basis of their morphology and immunophenotype, and the clinical records were reviewed.
Results—There were six primary lymphomas: three extranodal marginal zone lymphomas of mucosa associated lymphoid tissue (MALT) type and three diffuse large B cell lymphomas. Of the five secondary cases, four were diffuse large B cell lymphomas, one secondary to a systemic follicular follicle centre lymphoma, and one nodular sclerosis Hodgkins disease. Four patients with secondary lymphoma for whom follow up was available had died of disease within 13 months of diagnosis. Primary lymphomas followed a more indolent course. In one case, there was evidence of transformation from low grade MALT-type to diffuse large B cell lymphoma. The most common presenting symptom was haematuria. Cystoscopic appearances were of solid, sometimes necrotic tumours resembling transitional cell carcinoma, and in one case the tumours were multiple. These cases represented 0.2% of all bladder neoplasms.
Conclusions—Diffuse large B cell lymphoma and MALT-type lymphoma are the most common primary malignant lymphomas of the bladder. Lymphoepithelial lesions in MALT-type lymphoma involve transitional epithelium, and their presence in high grade lymphoma suggests a primary origin owing to transformation of low grade MALT-type lymphoma. Primary and secondary diffuse large B cell lymphomas of the bladder are histologically similar, but the prognosis of the former is favourable.
Key Words: bladder • lymphoma • mucosa associated lymphoid tissue lymphoma
doi:10.1136/jcp.53.6.458
PMCID: PMC1731210  PMID: 10911804
11.  Aggressive extranodal peripheral T-cell non-Hodgkin's lymphoma: A rare case report and review 
Non-Hodgkin's lymphoma (NHL) is primarily a disorder of lymph nodes, when limited primarily to extranodal sites it is referred to as primary extranodal lymphoma and accounts for 24-48% of all cases of NHL. The development of peripheral T-cell lymphoma is poorly understood and is thought to be associated with effector T cells involving chromosomal translocations. It has been suggested by few authors that the continued increased risk for NHL among highly active antiretroviral therapy (HAART)-treated human immunodeficiency virus (HIV)-infected persons may be a reflection of incomplete immune reconstitution and possibly a chronic state of B-cell hyperactivation. Here, we present one such rare case of extranodal NHL in a known HIV patient.
doi:10.4103/0973-029X.131918
PMCID: PMC4065453  PMID: 24959042
Extranodal NHL; HIV; non-Hodgkin's lymphoma
12.  Mantle cell lymphoma presenting as a breast mass 
Journal of Clinical Pathology  2001;54(11):883-886.
Breast lymphoma accounts for less than 1% of all non-Hodgkin's lymphomas (NHLs) and approximately 0.1% of all breast neoplasms. Most breast lymphomas are classified as diffuse large B cell or mucosa associated lymphoid tissue (MALT) lymphomas. The case of a 53 year old woman presenting with a breast mass and found to have mantle cell lymphoma is described. Core biopsy of the breast lesion showed a B cell NHL, probably of large cell type and of high grade. Morphological and immunophenotypic analysis of peripheral blood and bone marrow samples suggested a mantle cell lymphoma (MCL). This was confirmed by the detection of a t(11;14) in the bone marrow aspirate and breast tissue by polymerase chain reaction analysis. There have been no previous reports of an MCL presenting as a breast lump. Because a diagnosis of MCL has prognostic and therapeutic implications, this case highlights the need for an awareness of MCL presenting in this way, and the requirement for specialised investigations in its detection.
Key Words: mantle cell lymphoma • breast lymphoma • cyclin D1 • t(11;14)
PMCID: PMC1731308  PMID: 11684727
13.  Cytodiagnosis of the primary breast lymphoma in a young woman 
Primary breast lymphoma (PBL) is a rare entity. It represents 0.4% of malignant breast lesions and 2% of extranodal lymphomas. A 23-year-old woman presented with a left breast lump followed by palpable left axillary lymphadenopathy. Fine-needle aspiration cytology (FNAC) and histopathology were diagnostic of non-Hodgkin lymphoma-large cell type. Immunohistochemistry was positive against leukocyte common antigen and CD20. Because PBLs are uncommon malignant lesions and they usually do not have characteristic clinical and imaging findings, FNAC may prove to be a simple, rapid, reliable, and cost-effective procedure for successful diagnosis of the PBL.
doi:10.4103/0970-9371.126674
PMCID: PMC3945635  PMID: 24648678
Breast lymphoma; FNAC; immunohistochemistry
14.  Burkitt lymphoma masquerading as cardiac tamponade 
A 61 year old man presented with diffuse large B cell lymphoma of the skin of the back of the shoulder which was excised and treated with chemotherapy (CHOP regime) in 1998. He was in complete remission till he presented in 2002 with extranodal marginal zone lymphoma of the parotid gland for which he underwent superficial parotidectomy and radiotherapy. He continued in remission till 2006 when he presented with recurrent pericardial effusion and tamponade. At median sternotomy, pericardial effusion was drained, an anterior pericardiectomy was done and a left posterior pericardial window made, and an enlarged hard paraaortic lymph node excised. Histology, immunocytochemistry and chromosome analysis revealed Burkitt lymphoma. Patient underwent chemotherapy with CODOX-M regime and continues in remission. This report is unusual on account of the highly atypical presentation of Burkitt lymphoma as cardiac tamponade, only a few cases having been reported previously, the occurrence of three lymphomas of different pathological and genomic profiles in one patient over a period of eight years and the relatively slow rate of growth of an otherwise fulminant tumour with high tumour doubling time. A review of literature with special emphasis on chromosomal diagnosis, transformation of other lymphomas into Burkitt lymphoma and mediastinal and cardiac involvement with Burkitt lymphoma is presented.
doi:10.1186/1749-8090-2-30
PMCID: PMC1934902  PMID: 17615068
15.  Primary testicular lymphoma with rupture: An unusual presentation 
Primary testicular lymphoma usually presents as a unilateral testicular mass with occasional bilateral involvement. The tumor show contiguous spread to rete testis, epididymis spermatic cord and rarely to tunica albuginea. We report a case of primary testicular lymphoma which showed rupture of tunica albuginea with involvement of inguinal lymph node which is unusual. A 50-year-old male patient presented with right inguinal swelling and right side scrotal swelling of five months’ duration. Fine needle aspiration of the right inguinal lymph node was done and was suggestive for lymphoma/seminoma. Histopathology of right orchiectomy revealed non-Hodgkin's lymphoma (NHL). Further investigations did not reveal any other organs involved with non-Hodgkin's lymphoma. Primary testicular lymphoma usually shows spread to extranodal sites like skin, central nervous system and Waldeyer's ring at presentation and at relapse. Whereas, less common sites are lung, bone, liver, gastrointestinal system and nodal sites, especially the paraaortic lymph nodes. Testicular lymphoma with involvement of the inguinal lymph node is unusual. Clinical presentation of such cases may mimic germ cell tumors.
doi:10.4103/0976-9668.107314
PMCID: PMC3633286  PMID: 23633871
Lymphoma; primary; testis
16.  A Reappraisal of the Diagnostic and Therapeutic Management of Uncommon Histologies of Primary Ocular Adnexal Lymphoma 
The Oncologist  2013;18(7):876-884.
Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%–2% of non-Hodgkin lymphoma and 5%–15% of extranodal lymphoma. The most common and well-studied histotype is represented by marginal zone B-cell lymphoma. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management.
Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%–2% of non-Hodgkin lymphoma and 5%–15% of extranodal lymphoma. Histology, stage, and primary localizations are the most important variables influencing the natural history and therapeutic outcome of these malignancies. Among the various lymphoma variants that could arise in the ocular adnexa, marginal zone B-cell lymphoma (OA-MZL) is the most common one. Other types of lymphoma arise much more rarely in these anatomical sites; follicular lymphoma is the second most frequent histology, followed by diffuse large B-cell lymphoma and mantle cell lymphoma. Additional lymphoma entities, like T-cell/natural killer cell lymphomas and Burkitt lymphoma, only occasionally involve orbital structures. Because they are so rare, related literature mostly consists of anecdotal cases included within series focused on OA-MZL and sporadic case reports. This bias hampers a global approach to clinical and molecular properties of these types of lymphoma, with a low level of evidence supporting therapeutic options. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management.
doi:10.1634/theoncologist.2012-0425
PMCID: PMC3720642  PMID: 23814042
Ocular adnexal lymphoma; Diffuse large B-cell lymphoma; Follicular lymphoma; Mantle cell lymphoma; T-cell lymphoma
17.  Primary mucosa-associated lymphoid tissue thyroid lymphoma: a rare thyroid neoplasm of extrathyroid origin 
Rare Tumors  2012;4(1):e2.
Primary thyroid lymphoma is a rare malignancy, representing 2–8% of all thyroid malignancies and 1–2% of all extranodal lymphomas. The majority of cases concern non-Hodgkin's lymphoma of B cell origin, following by Hodgkin's disease, T cell lymphomas and rarely marginal zone B-cell mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas have been associated with long-standing autoimmune Hashimoto's thyroiditis. We present the case of a 44-years-old woman with thyroid MALT lymphoma in the background of multinodular goiter of autoimmune origin.
doi:10.4081/rt.2012.e2
PMCID: PMC3325747  PMID: 22532918
thyroid MALT lymphoma; thyroiditis Hashimoto.
18.  Primary T cell lymphoma of salivary gland: a report of a case and review of the literature 
Journal of Clinical Pathology  2002;55(1):61-63.
Lymphoma of the salivary gland accounts for 5% of cases of extranodal lymphoma and 10% of malignant salivary gland tumours. Most primary salivary gland lymphomas are B marginal zone lymphomas arising on a background of sialadenitis associated with autoimmune disorders such as Sjorgen's syndrome. Primary T cell lymphoma of the salivary gland is rare. This report describes a case of primary T cell lymphoma arising in the parotid gland of an elderly white man, which was notable for its striking resemblance to a B cell extranodal marginal zone lymphoma. Immunohistochemistry and gene rearrangement studies confirmed the clonal T cell nature of the tumour. There was no molecular evidence of Epstein-Barr virus (EBV) infection of neoplastic or surroundings cells. Only 14 cases of primary T cell lymphoma of the salivary glands have been recorded in the literature, most being from the Orient and having extremely variable prognosis. Those with a T/natural killer cell phenotype are associated with EBV infection. This case highlights the fact that T cell lymphoma in the salivary gland can mimic closely the morphological features of B cell extranodal marginal zone lymphoma.
PMCID: PMC1769572  PMID: 11825927
salivary gland; T cell lymphoma; Epstein Barr virus
19.  Treatment options for ocular adnexal lymphoma (OAL) 
Most lymphomas that involve the ocular adnexal structure are low grade, B cell, non-Hodgkin’s lymphomas. The treatment depends upon the grade and stage of the disease. High grade lymhoma requires treatment with systemic chemotherapy whereas the localized low grade (extranodal marginal zone lymphoma) can be successfully managed with local radiotherapy. Chlamydia psittaci infection is associated with low grade ocular lymphoma; however there is wide geographic variation in the strength of this association. Blanket antibiotic therapy is not advised unless there is proof of an infective agent. The monoclonal antibody, rituximab, may be successful for CD20 positive lymphoma, although it is likely that rituximab will have better long-term results when used in combination with systemic chemotherapy.
PMCID: PMC2801639  PMID: 20054418
ocular adnexal lymphoma; mucosa associated lymphoid tissue; extranodal marginal zone lymphoma; Chlamydia psittaci; rituximab; radiotherapy; chemotherapy
20.  Subcutaneous panniculitis-like T-cell lymphoma in a case of carcinoma cervix 
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon extra nodal non-Hodgkin lymphoma accounting for less than 1% of all NHLs known to have an aggressive course, with no well-defined treatment protocols. A 42-year-old lady, operated five months earlier for a squamous cell carcinoma of the cervix presented with pain and induration of the lower part of the anterior abdominal wall; 3 months after completing chemotherapy and radiotherapy. FNAC done, yielded scanty material and was inconclusive. The biopsy showed features of a subcutaneous panniculitis-like T-cell lymphoma. While on chemotherapy she developed a vault recurrence and extensive intra-abdominal spread of the squamous cell carcinoma and succumbed. SPTCL is a rare entity and has been reported in renal and cardiac allograft recipients and in one case of ovarian carcinoma. Its occurrence in the setting of carcinoma cervix is unusual, hence is being reported.
doi:10.4103/0971-5851.116192
PMCID: PMC3764727  PMID: 24049298
Carcinoma cervix; panniculitis; T-cell lymphoma
21.  Radiotherapy in marginal zone lymphoma 
Purpose
To evaluate the efficacy of radiotherapy (RT) for early-stage nodal and extranodal marginal zone lymphoma (MZL).
Materials and methods
Patients with stage I (n = 22) and stage II (n = 8) MZL, who were treated with RT were reviewed. The primary tumor localisation was in the orbita (n = 12), stomach (n = 8), head and neck other than the orbita (n = 8), breast (n = 1) and one case of marginal zone lymphoma of the skin (n = 1). The median radiotherapy dose was 40 Gy (5 to 45 Gy).
Results
The median follow-up time was 103 months. The 5-year overall survival and event-free survival rates were 85 ± 7% and 71 ± 9%, respectively. There was no infield recurrence. Recurrence occurred outside of the radiation field in six patients. The relapses were treated with salvage RT and had excellent local control (100%) at five years after salvage RT.
Conclusions
Localized extranodal MZL have an excellent prognosis following moderate-dose RT. RT is also an effective salvage therapy in cases of localized recurrence. Further clinical studies should evaluate the optimal dose for MZL.
doi:10.1186/1748-717X-8-2
PMCID: PMC3561230  PMID: 23281682
Gastric lymphoma; MALT; Marginal zone lymphoma; Radiotherapy
22.  A rare case of breast carcinoma co-existing with axillary mantle cell lymphoma 
Background
Mantle cell lymphoma (MCL) is a rare variety of non-Hodgkin's lymphoma which originates from CD5+ B-cell population in the mantle zones of lymphoid follicles. Coexistence of such tumours in the axillary lymph nodes with invasive breast cancers without prior history of adjuvant chemotherapy or radiotherapy has not been previously reported in literature.
Case report
We report a rare case of breast cancer co-existing with stage I mantle cell lymphoma of the ipsilateral axillary lymph node detected fortuitously by population screening.
Conclusion
Though some studies have tried to prove breast carcinomas and lymphomas to share a common molecular or viral link, more research needs to be done to establish whether such a link truly exists.
doi:10.1186/1477-7819-1-27
PMCID: PMC317372  PMID: 14664721
Lymphoma; breast; axilla; cancer; mantle cell; B-cell; carcinoma; neoplasm; lymph node; non Hodgkin's lymphoma
23.  Primary pediatric gastrointestinal lymphoma 
Background:
Primary non-Hodgkin's lymphoma (NHL) of the gastrointestinal (GI) tract is the most common extranodal lymphoma in pediatric age group. Yet, the overall incidence is very low. The rarity of the disease as well as variable clinical presentation prevents early detection when the possibility of cure exists.
Materials and Methods:
We studied six cases of primary GI NHL in pediatric age group with reference to their clinical presentation, anatomic distribution and histopathologic characteristics.
Results:
All were males except one. Intestinal obstruction was the presenting feature in 50%. Half the cases showed ileocaecal involvement, while large bowel was involved in 16%. Histology showed four cases of diffuse large B-cell lymphoma (DLBCL), one case of Burkitt lymphoma, and one Burkitt-like lymphoma. Immunohistochemistry for Tdt, CD20, CD3, CD30, bcl2, bcl6 confirmed the morphological diagnosis.
Conclusion:
Pediatric GI lymphoma commonly involves the ileocaecal region and presents with intestinal obstruction. A higher prevalence of DLBCL is found compared to other series. A high proliferative index is useful in differentiating Burkitt-like lymphoma from DLBCL.
doi:10.4103/0971-5851.89786
PMCID: PMC3237187  PMID: 22174497
Gastrointestinal tract; non-Hodgkin's lymphoma; pediatric
24.  Gastric Mucosa-Associated Lymphoid Tissue Lymphoma Followed by Primary Central Nervous System Lymphoma 
Mucosa-associated lymphoid tissue (MALT) lymphoma is one of the most common lymphomas and accounts for about 7% of all newly diagnosed non-Hodgkin's lymphoma (NHL). The clinical course of MALT lymphoma is relatively indolent and, in the majority of cases (50%), the lymphoma arises within the stomach. Primary central nervous system lymphoma (PCNSL), an uncommon variant of extranodal NHL, can affect any part of the neuraxis, including the eyes, brain, leptomeninges, or spinal cord. Herein, we present a rare case of PCNSL, which occurred one year after radiochemotherapy of gastric MALT lymphoma. A 62-year-old man presented with a 3-day history of left facial palsy. One year ago, he underwent antibiotic eradication therapy of Helicobacter pylori, local stomach fractional radiotherapy, and chemotherapy for gastric MALT lymphoma. Magnetic resonance imaging revealed a strong enhancing solid mass in the right frontal lobe. The tumor was completely removed, and the histological diagnosis of PCNSL developing from diffuse large B-cell lymphoma was made. Although elucidating the correlation between the first gastric MALT lymphoma and the second PCNSL seemed difficult, we have postulated and discussed some possible pathogeneses, together with a review of literature.
doi:10.3340/jkns.2012.51.6.377
PMCID: PMC3424181  PMID: 22949970
MALT Lymphoma; Diffuse large B-Cell lymphoma
25.  Malignant lymphomas of the nasal cavity and Waldeyer's ring--clinicopathologic and immunohistochemical study. 
The clinicopathologic and immunohistochemical finding of 10 cases of nasal non-Hodgkin's lymphoma (NHL) and 23 cases of Waldeyer's ring NHL were studied. Immunohistochemically, nasal NHL expressed T-cell markers exclusively, whereas the NHL of Waldeyer's ring were of both T-cell (56.5%) and B-cell lineages (43.5%). Angioinvasiveness by tumor cells was exclusively noted in the T-lineage lymphomas. Epithelial hyperplasia, epitheliotropism by tumor cells, and extensive invasion of adjacent normal tissue were more prominent in T-cell lymphomas than in B-cell lymphomas. T-lineage lymphomas showed distant extranodal spread pattern involving the skin, soft tissue, stomach, spleen, and the liver, whereas B-lineage lymphomas tended to localize in the lymph nodes. The survival rate of Nasal NHL was similar to that of Waldeyer's ring NHL. Although not statistically significant because of small sample numbers, immunophenotype, histologic groups of monomorphic lymphoma, and stage had prognostic importance. In general, T-lineage lymphomas presented with a higher stage than B-lineage lymphomas (p < 0.05)-and overall survival was poor. Stage I disease showed a much more favorable prognosis than stage II disease. Monomorphic lymphomas had a shorter survival than polymorphic reticulosis (PR) or lymphomas with features of PR. This result in conjunction with the morphologic transition between them suggested that monomorphic lymphoma may represent the most advanced stage in the spectrum of PR, lymphoma with features of PR, and monomorphic lymphoma.
PMCID: PMC3053843  PMID: 1299234

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